Imaging in Propeller Flap Surgery.

Since propeller flaps are elevated as island flaps and most often nourished by a single perforator nearby the defect, it is challenging to change the flap design intraoperatively when a reliable perforator cannot be found where expected to exist. Thus, accurate preoperative mapping of perforators is essential in the safe planning of propeller flaps. Various methods have been reported so far: (1) handheld acoustic Doppler sonography (ADS), (2) color duplex sonography (CDS), (3) perforator computed tomographic angiography (P-CTA), and (4) magnetic resonance angiography (MRA). To facilitate the preoperative perforator assessment, P-CTA is currently considered as the gold standard imaging tool in revealing the three-dimensional anatomical details of perforators precisely. Nevertheless, ADS remains the most widely used tool due to its low cost, faster learning, and ease of use despite an undesirable number of false-positive results. CDS can provide hemodynamic characteristics of the perforator and is a valid and safer alternative particularly in patients in whom ionizing radiation and/or contrast exposure should be limited. Although MRA is less accurate in detecting smaller perforators of caliber less than 1.0 mm and the intramuscular course of perforators at the present time, MRA is expected to improve in the future due to the recent developments in technology, making it as accurate as P-CTA. Moreover, it provides the advantage of being radiation-free with fewer contrast reactions.

Efficacy and safety of novel collagen conduits filled with collagen filaments to treat patients with peripheral nerve injury: A multicenter, controlled, open-label clinical trial.

INTRODUCTION: The safety and efficacy of using artificial collagen nerve conduits filled with collagen filaments to treat nerve defects has not been fully studied in humans. We conducted a multicenter, controlled, open-label study to compare the safety and efficacy of artificial nerve conduit grafts with those of autologous nerve grafts. METHODS: We included patients with a sensory nerve defect of ≤30 mm, at the level of the wrist or a more distal location, with the first-line surgical methods selected according to a patient's preference. We compared sensory recovery using static two-point discrimination and adverse events between the artificial collagen nerve conduit and autologous nerve grafting. RESULTS: The artificial nerve conduit group included 49 patients, with a mean age of 42 years and nerve defect of 12.6 mm. The autologous nerve graft group included 7 patients, with historical data of an additional 31 patients, with a mean age of 36 years and nerve defect of 18.7 mm. The rate of recovery of sensory function at 12 months was 75% (36/49) for the artificial nerve conduit group and 73.7% (28/38) in the autologous nerve group. No serious adverse events directly associated with use of the artificial nerve conduit were identified. CONCLUSIONS: The treatment of nerve defects ≤30 mm using artificial collagen nerve conduits was not inferior to treatment using autologous nerve grafts. Based on our data, the new artificial collagen nerve conduit can provide an alternative to autologous nerve for the treatment of peripheral nerve defects.

Microsurgical Flaps in Repair and Reconstruction of the Hand.

The authors' strategy for soft-tissue coverage of the hand is presented. The concept of replacing like with like and reconstruction with similar adjacent tissue enhances functional and aesthetic outcomes. In this viewpoint, the pedicle perforator flap is an ideal flap. A decision-making algorithm to select an ideal flap for a particular hand defect is challenging, requiring experiential consideration of functional outcome, appearance, donor-site morbidity, and patient satisfaction. To assist surgeons in determining the most appropriate flap with more evidence, studies are necessary to compare the outcomes of each flap by evaluating hand function, aesthetics, donor site morbidity, and patient satisfaction.

Imaging Studies for Preoperative Planning of Perforator Flaps: An Overview.

The vascular anatomy of perforators varies between individuals; thus, accurate preoperative assessment of perforators is essential for safely planning perforator flaps. Perforator computed tomographic angiography (P-CTA) with multidetector-row computed tomography (MDCT) is one of the best available methods to precisely reveal the 3-dimensional anatomic details of perforators. The aim of this report is to describe the authors' experience using P-CTA with MDCT for detecting the perforating vessel preoperatively and a step-by-step approach to harvest perforator flaps based on this technique. This report also provides a comprehensive review of literature on other preoperative assessment tools of perforators.

Reliability and Validity of the Japanese Version of the Michigan Hand Outcomes Questionnaire: A Comparison with the DASH and SF-36 Questionnaires.

BACKGROUND: The Michigan Hand Outcomes Questionnaire (MHQ) has shown reliability, validity and responsiveness and has been used to assess surgical outcomes mainly in North America. We established a Japanese version of the MHQ and evaluated its reliability and validity compared with both the short-form 36 (SF-36) questionnaire and the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire in a Japanese-speaking population. METHODS: The MHQ was cross-culturally adapted to a Japanese version according to guidelines. Sixty-eight patients with hand conditions were enrolled in this study and answered the MHQ, DASH questionnaire and SF-36 questionnaire. The MHQ was completed again with an interval of one or two weeks. Reproducibility and internal consistency were statistically assessed by the test-retest method and calculating Cronbach's alpha. Spearman's rank correlation was calculated to assess associations between the MHQ and the SF-36 questionnaire as well as the DASH questionnaire. RESULTS: The intraclass correlation coefficients of MHQ subscales ranged from 0.68 to 0.93. Aesthetics subscale of the left hand showed the lowest intraclass correlation but still a good correlation. Cronbach's alpha values of the MHQ ranged from 0.81 to 0.96 for all subscales. The absolute values of Spearman's rank correlation coefficient between MHQ subscales and DASH function/symptoms scores ranged from 0.49 to 0.82. Spearman's correlation coefficients of the MHQ total score to subscales of the SF-36 questionnaire ranged from 0.42 to 0.68. The strongest correlations were found between work performance of the MHQ and rolephysical of the SF-36 questionnaire. CONCLUSIONS: The Japanese version of the MHQ has adequate instrument properties for assessing hand outcomes compared with the SF-36 questionnaire as well as the DASH questionnaire.

Hepatic arterial infusion chemotherapy using fluorouracil, epirubicin, and mitomycin C for patients with liver metastases from gastric cancer after treatment failure of systemic S-1 plus cisplatin.

BACKGROUND: For patients with liver metastases from gastric cancer (LMGC), combination chemotherapy with fluoropyrimidines and platinum agents has been recognized as standard treatment. However, the prognosis of hepatic progression after first-line treatment failure remains poor. When hepatic progression occurs, hepatic arterial infusion (HAI) chemotherapy may be helpful for preventing disease progression. PURPOSE: To retrospectively assess the feasibility and efficacy of HAI chemotherapy using 5-fluorouracil, epirubicin, and mitomycin C (FEM) for patients with LMGC after failure of systemic S-1 plus cisplatin. MATERIAL AND METHODS: We reviewed the records of patients who received HAI chemotherapy using FEM for LMGC that progressed during systemic S-1 plus cisplatin treatment while extrahepatic disease was decreased or did not appear. HAI chemotherapy was given as second-line therapy using 5-fluorouracil (330 mg/m(2) weekly), epirubicin (30 or 40 mg/m(2) every 4 weeks), and mitomycin C (2.7 mg/m(2) biweekly). RESULTS: Fourteen patients were analyzed. Toxicity of HAI chemotherapy was generally mild. The objective response rate was 42.9%, including a complete response rate of 14.3%. Median times to hepatic and extrahepatic progression were 9.2 and 7.4 months, respectively. Of 12 patients with documented progression after HAI chemotherapy, 10 patients (83.3%) received additional treatment, including irinotecan or taxanes. Overall, median survival was 12.7 months. CONCLUSION: Our findings suggest that HAI chemotherapy using FEM is a feasible and effective treatment for patients with LMGC after failure of systemic S-1 plus cisplatin. HAI chemotherapy employed in the second-line setting is useful for achieving long-term disease control of LMGC.

Leukocytosis and high hematocrit levels during abdominal attacks of hereditary angioedema.

BACKGROUND: The diagnosis of hereditary angioedema (HAE) is often delayed due to the low awareness of this condition. In patients with undiagnosed HAE, abdominal symptoms often create the risk of unnecessary surgical operation and/or drug therapy. To explore the cause of misdiagnosis, we compared the laboratory findings of HAE patients under normal conditions with those during abdominal attacks. METHODS: Patient medical histories were analyzed and laboratory data at the first consultation with no symptoms and no medication were compared with those at visits to the emergency department during severe attacks. RESULTS: Fourteen HAE patients were enrolled. Initial HAE symptoms occurred at 20.2 ± 9.4 years of age. The correct diagnosis of HAE was made 22.7 ± 14.2 years after the initial symptoms. A common site of angioedema was the extremities. Half of the patients experienced a life-threatening laryngeal attack and/or severe abdominal pain. In the patients with severe abdominal pain, significant leukocytosis with neutrophilia along with increased levels of hematocrit were observed while levels of C-reactive protein (CRP) remained low. All severe attacks were alleviated with an infusion of C1-inhibitor concentrate. CONCLUSIONS: Consideration of the likelihood of a HAE attack is important when patients present with acute abdominal pain and leukocytosis without elevation of CRP.

Reliability, validity, and responsiveness of the Japanese version of the patient-rated elbow evaluation.

BACKGROUND: The patient-rated elbow evaluation (PREE) is a joint-specific, self-administered questionnaire consisting of a pain scale (PREE-P) and a functional scale (PREE-F), the latter consisting of specific function (PREE-SF) and usual function (PREE-UF). The purpose of this study was to cross-culturally adapt the PREE into Japanese (PREE-J) and to test its reliability, validity, and responsiveness. METHODS: A consecutive series of 74 patients with elbow disorder completed the PREE-J, the Japanese version of the disabilities of the arm, shoulder, and hand (DASH-JSSH) questionnaire, and the official Japanese version of the 36-Item Short-Form Health Survey (SF-36). Of the 74 patients, 53 were reassessed for test-retest reliability 1 or 2 weeks later. Reliability was investigated in terms of reproducibility and internal consistency. The validity of the PREE-J was examined by factor analysis, and correlation coefficients were obtained using the PREE-J, DASH-JSSH, and SF-36. Responsiveness was examined by calculating the standardized response mean (SRM) and effect size after elbow surgery in 53 patients. RESULTS: Cronbach's α coefficients for PREE-P, PREE-F, and PREE were 0.92, 0.97, and 0.97, respectively, and the corresponding intraclass correlation coefficients were 0.92, 0.93, and 0.94, respectively. Unidimensionality of PREE-P and PREE-F was confirmed by factor analysis. The coefficients of correlation between PREE-P and PREE-F or DASH-JSSH were 0.81 and 0.74, respectively; that between PREE-F and DASH-JSSH was 0.86, and those between DASH-JSSH and PREE-SF or PREE-UF were 0.85 and 0.82, respectively. Moderate correlation was observed in "physical functioning" for SF-36 and PREE-F (r = -0.69) or PREE (r = -0.68). The SRMs/effect sizes of PREE-P (1.31/1.32) or PREE (1.28/1.12) were more responsive than the DASH-JSSH (0.99/0.85), "bodily pain" (-1.15/-1.43), and "physical functioning" (-0.70/-0.44) in SF-36. CONCLUSION: The PREE-J represents a reliable, valid, and responsive instrument and has evaluation capacities equivalent to those of the original PREE.

Fluctuation of serum C3 levels reflects disease activity and metabolic background in patients with IgA nephropathy.

BACKGROUND: We focused on the fluctuations of serum C3 levels throughout the clinical course of patients and investigated the relationship between these fluctuations and clinical findings. METHODS: IgA nephropathy patients (n = 122) were enrolled in the present study. Serum C3 and other clinical markers were compared at the time of renal biopsy and at last follow-up (6.67 ± 2.07 years). Patients were divided into 3 groups based on serum C3 levels: Group I with first C3 levels below the mean -1 SD, which turned into an increase at last observation; group II with first C3 levels more than the mean +1 SD, which turned into a decrease at last observation; and group III, with first C3 levels more than the mean +1 SD, which turned into an increase at last observation. First and last levels of clinical markers were compared among the 3 groups. RESULTS: Serum C3 levels of the patients whose renal symptoms, including hematuria, proteinuria and estimated glomerular filtration rate (eGFR), were improved, were significantly increased at last observation (p<0.05, p<0.01, p<0.01, respectively). Age, total cholesterol and triglyceride levels in group III were significantly higher than those in group I. Group II showed a significant reduction of urinary protein. Groups I and II maintained renal function, but group III showed a significant deterioration of renal function. CONCLUSIONS: The levels and fluctuations of serum C3 might reflect the disease activity and metabolic alteration in patients with IgA nephropathy.

Esophageal cancer with an esophagopericardial fistula and purulent pericarditis.

We herein present the case of a 56-year-old Japanese woman who developed purulent pericarditis after undergoing chemoradiotherapy for esophageal cancer. She developed epigastralgia and a fever and was admitted to our hospital. A physical examination revealed hypotension, tachycardia and pericardial friction rub. Echocardiography revealed moderate pericardial effusion. Based on these observations, the patient was diagnosed with cardiac tamponade. Computed tomography confirmed the presence of an esophagopericardial fistula. Treatment with pericardiocentesis, drainage and short-term intrapericardial administration of antibiotics relieved the patient's symptoms. Daily rinsing through a catheter with normal saline prevented relapse of the purulent pericarditis. Esophagopericardial fistulas are so rare that their treatment is not well-established. We herein report successful palliative care of a malignant esophagopericardial fistula associated with purulent pericarditis.

Extraglomerular C3 deposition and metabolic impacts in patients with IgA nephropathy.

BACKGROUND: The aim of the present study was to explore the significance of extraglomerular (Bowman's capsule and/or arteriole) C3 (ex-C3) deposits in IgA nephropathy (IgAN). METHODS: One hundred and seventy patients with IgAN were divided into two groups: Group A (n=79), patients who did not have ex-C3 deposits, and Group B (n=91), patients who had ex-C3 deposits. RESULTS: At the time of renal biopsy, Group B was characterized by a marked increase in diastolic blood pressure, total cholesterol, triglyceride and low-density lipoprotein-cholesterol compared with those of Group A. After 4 years, the estimated glomerular filtration rate (eGFR) in Group B was significantly worse than that of Group A. Upon examination by electron microscopy, the arteriolar dense deposits in Group B were found to occur in significantly higher amounts than in Group A. One hundred and thirty-four patients underwent a 3-year follow-up study after intervention and were re-divided by therapeutic factors as follows: 'conventional therapy', treatment with anti-hypertensive drugs and/or anti-platelet drugs, and 'aggressive therapy', additional treatment with either tonsillectomy or corticosteroid. Patients treated with conventional therapy in Group B had significantly higher body mass index and levels of C3 and CH50 compared with other Groups. Aggressive therapy was significantly effective in urinary protein reduction in both Group A and Group B. Except for the patients who received aggressive therapy in Group A, the levels of the eGFR gradually declined. CONCLUSIONS: It appears that IgAN patients who have ex-C3 deposits have worse clinical outcomes.

[An approach to research on nephritic factor and membranoproliferative glomerulonephritis].

Although it has been 45 years since membranoproliferative glomerulonephritis (MPGN) and C3 nephritic factor (3NeF) were first reported, the pathophysiology of MPGN is not fully understood at present. Careful analysis of previous case reports of MPGN has been the key to developing approaches to study the mechanisms of MPGN pathophysiology. In this review, previous studies on MPGN, mainly on its association with C3NeF, are discussed and important issues on MPGN as yet unknown are stated. Complements play important roles in MPGN. Studies on complements, particularly C3NeF, advanced as studies on pathophysiology of MPGN progressed and consequently, complement activation alternative pathways were clarified. Important, although not well-known research subjects on MPGN, include characteristics of several kinds of NeF and type I, III and dense deposit disease (DDD). Detailed reading of case reports often yields new research ideas and study directions of the disease and its treatment. The present review demonstrates that reviewing previous case reports is one approach to further advancing our understanding of the complicated disease of MPGN.

Guideline for hereditary angioedema (HAE) 2010 by the Japanese Association for Complement Research - secondary publication.

This guideline was provided by the Japanese Association for Complement Research targeting clinicians for making an accurate diagnosis of hereditary angioedema (HAE), and for prompt treatment of the HAE patient in Japan. This is a 2010 year version and will be updated according to any pertinent medical advancements.

Determination of severity of murine IgA nephropathy by glomerular complement activation by aberrantly glycosylated IgA and immune complexes.

The pathogenic roles of glomerular deposition of components of the complement cascade in IgA nephropathy (IgAN) are not completely clarified. To investigate the pathologic role of complement pathways in IgAN, two IgAN-prone mouse models were examined. Grouped ddY (gddY) mice showed significant high proteinuria, severe glomerular lesions, and extracellular matrix expansion compared with high serum IgA (HIGA) mice but with similar intensity of glomerular IgA deposition. Glomerular activation of the classical, lectin, and alternative pathways was demonstrated by significantly stronger staining for complement (C)3, C5b-9, C1q, C4, mannose-binding lectin (MBL)-A/C, MBL-associated serine protease-2, and factor B and properdin in gddY mice than in HIGA mice. Similarly, the serum levels of IgA-IgG2a/IgM and IgA-MBL-A/C immune complexes and polymeric IgA were significantly higher in gddY mice than in HIGA mice. Moreover, the serum levels of aberrantly glycosylated IgA characterized by the binding of Sambucus nigra bark lectin and Ricinus communis agglutinin I were significantly higher in gddY mice than in HIGA mice. This aberrancy in glycosylation was confirmed by monosaccharide compositional analysis of purified IgA using gas-liquid chromatography. This study is the first to demonstrate that aberrantly glycosylated IgA may influence the formation of macromolecular IgA including IgA-IgG immune complexes and subsequent complement activation, leading to full progression of IgAN.

Pathological scenario with the mannose-binding lectin in patients with IgA nephropathy.

A deeper understanding of the mechanism of complement activation may help to elucidate the pathogenesis of IgA nephropathy (IgAN). Traditionally, the activation of an alternative pathway (AP) has been recognized as an enhancer mechanism of glomerular damage. This paper documents contemporary information concerning the possible pathological mechanisms of the lectin pathway (LP) in the circulation and in the glomerulus. The circulating initiator of LP activation is not fully understood. However, ligands for mannose-binding lectin (MBL) which are among the starter molecules of the LP are aberrant glycosylated molecules-containing immune complex. Recent reports have focused on N-glycans on secretory IgA as a candidate ligand. Mesangial deposits of MBL are seen in 25% of patients with IgAN. Mesangial deposits of MBL and C4 and/or C4 breakdown products are implicated as markers for disease progression of IgAN. On the other hand, patients with MBL deficiency tend to show better clinical presentation and lower levels of urinary protein and serum creatinine than MBL-sufficient patients. It is now recognized that involvement of AP and LP constitutes an additional mechanism for explaining the progression of IgAN.

Significance of broad distribution of electron-dense deposits in patients with IgA nephropathy.

Immunoglobulin A nephropathy (IgAN) is characterized by mesangial cell proliferation and mesangial expansion with mesangial depositions of IgA. We have found that electron-dense deposits (EDD) are often observed in areas other than paramesangial areas in glomeruli. To compare electron microscopic findings with light microscopic findings and clinical data, we examined the biopsies from 178 patients with IgAN. Patients were divided into two groups: group A had only paramesangial deposits and group B had deposits not only in paramesangial areas but also in other areas. All patients examined in this study had EDD in glomerular paramesangial areas. Thirty-six patients were included in group B. Cellular crescent formation in glomeruli and urinary protein in group B were significantly higher than those in group A (P < 0.01). Serum albumin and estimated glomerular filtration rate (eGFR) in group B were significantly lower than those in group A (P < 0.05). Group B showed a significant positive correlation with histological severity, which is defined in the Japanese Clinical Guidelines on IgAN. In patients with broad distribution of EDD, urinary protein was significantly increased (P < 0.05). Detailed observation of EDD distribution has an impact on evaluation of the disease activity of IgAN.

An analysis of functional activity via the three complement pathways during hemodialysis sessions: a new insight into the association between the lectin pathway and C5 activation.

BACKGROUND: We have recently demonstrated that hemodialysis (HD) patients have significantly higher levels of functional complement activity (FCA) in all three pathways, i.e. the classical pathway, alternative pathway and lectin pathway (LP), than in age-matched controls, though the role of FCA during HD still remains unknown. METHODS: Serial plasma or serum samples were obtained from five patients during HD in order to investigate the kinetics of complement components. The levels of the C5b-9 complex, the FCA of the three pathways, a derivative of C3a (C3a desArg) and a derivative of C5a (C5a desArg) in the samples were analyzed. RESULTS: The levels of the C5b-9 complex at 60 min were significantly increased when compared with those at 0 min. Functional activities for all three pathways showed different patterns so the same tendency between pathways was not observed. The levels of C3a desArg and C5a desArg at 60 min were markedly increased when compared with those at 0 min. A Spearman's rho test showed a strong positive correlation between functional LP activity and C5a desArg. CONCLUSIONS: These findings lead to new insights into the FCA during HD and suggest that functional LP activity has an important role in C5 activation.

Locking palmar plate fixation for dorsally displaced fractures of the distal radius: a preliminary report.

We reviewed a series of 62 consecutive patients with dorsally displaced fractures of the distal radius, including 20 extra-articular and 42 intra-articular fractures. All patients were treated with palmar locking plate systems at our institution between 2002 and 2006. After a minimum follow-up time of 12 weeks, the fractures had healed with satisfactory radiographic and functional results. According to the demerit point system of Gartland and Werley, 35 patients were rated excellent, 26 good, and one fair. In the good and fair groups, the demerit points were almost all for ulnar wrist pain. Our results suggest that palmar locking plate systems enable early functional mobilization with good reproducible radiographic and clinical outcomes. Since nine out of 62 patients had residual ulnar wrist pain at the final follow-up evaluation, further investigation of the pathogenesis of ulnar wrist pain is necessary to obtain better functional outcomes.

Excretion of complement proteins and its activation marker C5b-9 in IgA nephropathy in relation to renal function.

BACKGROUND: Glomerular damage in IgA nephropathy (IgAN) is mediated by complement activation via the alternative and lectin pathways. Therefore, we focused on molecules stabilizing and regulating the alternative pathway C3 convertase in urine which might be associated with IgAN pathogenesis. METHODS: Membrane attack complex (MAC), properdin (P), factor H (fH) and Complement receptor type 1 (CR1) were quantified in urine samples from 71 patients with IgAN and 72 healthy controls. Glomerular deposition of C5, fH and P was assessed using an immunofluorescence technique and correlated with histological severity of IgAN and clinical parameters. Fibrotic changes and glomerular sclerosis were evaluated in renal biopsy specimens. RESULTS: Immunofluorescence studies revealed glomerular depositions of C5, fH and P in patients with IgAN. Urinary MAC, fH and P levels in IgAN patients were significantly higher than those in healthy controls (p < 0.001), but CR1 was significantly lower than that in healthy controls (p < 0.001). Urinary MAC and fH levels were positively correlated with serum creatinine (sCr), urinary N-acetyl-ß-D-glucosaminidase (u-NAG), urinary ß2 microglobulin (u-Bm), urinary protein (p < 0.001), interstitial fibrosis (MAC: p < 0.01, fH: p < 0.05) and the percentage of global glomerular sclerosis (p < 0.01). Urinary P was positively correlated with u-NAG, u-Bm, and urinary protein (p < 0.01). CONCLUSIONS: Complement activation occurs in the urinary space in IgAN and the measurement of levels of MAC and fH in the urine could be a useful indicator of renal injury in patients with IgAN.

Serum concentration of complement components of the lectin pathway in maintenance hemodialysis patients, and relatively higher levels of L-Ficolin and MASP-2 in Mannose-binding lectin deficiency.

Mannose-binding lectin (MBL), L-ficolin and MBL associated serine protease-2 (MASP-2) are molecules involved in initiation of the lectin pathway (LP) in the complement system. Although MBL deficiency is observed in almost 10% of healthy people, studies of associations between MBL deficiency and end-stage renal disease (ESRD) remain rare. The objective of the present study is to clarify the significance of the LP in maintenance hemodialysis (HD) patients, especially in terms of MBL levels. Two hundred and forty-four HD patients who had been followed up for 74±84months and 199 healthy controls were included in this study. Measurements of serum concentrations of MBL, L-ficolin, and MASP-2 were performed. Low serum MBL levels (<0.1µg/mL) in the patients were confirmed by examination of a point mutation in the Mbl-2 gene. Seventeen HD patients (7%) and 20 healthy controls (10%) had MBL deficiency. During the follow-up period, 99 patients died. There was no significant difference in the frequency of deaths by infectious diseases between MBL deficient and non-deficient patients. In both patients and healthy controls with MBL deficiency, the serum concentration of L-ficolin tended to be high, and that of MASP-2 was significantly high (P<0.05). MBL deficiency is not a risk factor for HD induction or life-threatening infections. It is postulated that the elevation of concentration of the two components of the LP, L-ficolin and MASP-2, may compensate for the insufficient activity of the LP in MBL deficiency.